- Superficial malignant peripheral nerve sheath tumor from recurrent neurofibroma in the abdominal wall of a patient without neurofibromatosis type 1
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Chang Yeon Jung, Jung Min Bae, Joon Hyuk Choi, Ki Hoon Jung
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Yeungnam Univ J Med. 2019;36(1):63-66. Published online December 20, 2018
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DOI: https://doi.org/10.12701/yujm.2019.00031
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Abstract
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- Malignant peripheral nerve sheath tumor (MPNST) is rare, accounting for 5–10% of all soft tissue sarcomas. MPNST is characteristically aggressive and has a poor prognosis. Fifty percent of patients with MPNST have neurofibromatosis type 1 (NF1). NF-associated MPNST (NF-MPNST) occurs more often at younger ages than sporadic MPNST (sMPNST), but the survival difference is controversial. Superficial MPNST from a recurrent neurofibroma is extremely rare and only a limited number of cases have been reported in the literature. Herein, we report an unusual case of superficial MPNST from a recurrent neurofibroma in a patient without NF1.
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